以中耳炎为首发表现的抗中性粒细胞胞浆抗体相关血管炎临床特征

Otitis media as initial manifestation of antineutrophil cytoplasmic antibody-associated vasculitis

林颖;乔燕;杨润琴;徐卓;查定军

1:空军军医大学第一附属医院耳鼻咽喉头颈外科

摘要
目的 探讨抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibody, ANCA)相关血管炎性中耳炎(otitis media with ANCA associated vasculitis, OMAAV)的临床特点及诊治经验。方法 以日本耳科学会指南标准,对2015年9月至2022年9月诊断为OMAAV的19例患者的临床资料进行回顾性分析,包括一般资料、临床表现、病理学、影像学及血清免疫指标。结果 19例患者年龄46~76岁,平均61.8岁,免疫学检测指标:9例cANCA和蛋白酶3(predominantly proteinase 3,PR3)阳性(47.3%)、6例pANCA和髓过氧化物酶(myeloperoxidase, MPO)阳性(31.6%)、3例pANCA阳性、1例cANCA阳性。血管炎活动性评分平均6.6分,伴发受累组织:肺84.2%(16/19)、鼻73.7%(14/19)、面神经21.0%(4/19);9例患者行病理活检,2例提示ANCA相关疾病,余为非特异性炎症。所有患耳确诊时呈混合性聋,从出现耳科症状到确诊平均需14.2月,10耳曾接受鼓膜置管治疗;听力学随访资料完整的8例14耳,经单纯激素或联合免疫抑制剂治疗,耳科症状均有改善:治疗前后气导平均阈值分别为75.1±23.2、50.5±22.1 dB HL,差异有统计意义。结论 OMAAV多见于中老年患者,早期临床症状仅表现为难以治愈的分泌性中耳炎,易误诊;随病程进展骨导听力下降并出现伴发疾病,最常受累组织为肺和鼻,血清免疫学检验是重要的确诊依据;如果治疗不及时听力干预效果不佳。
关键词
中耳炎;抗中性粒细胞胞浆抗体;自身免疫性疾病
基金项目(Foundation):
作者
林颖;乔燕;杨润琴;徐卓;查定军
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